[ARVD-FORUM] Pathological difficulties to distinguish lipomatosis from true ARVD. Dr Basso

[ARVD Symposium]

English - Spanish

Dear Dr  Lorin de la Grandmaison,
I suggest you to read carefully the paper we recently published:
Basso C, Thiene G.  Adipositas cordis, fatty infiltration of the right
ventricle, and arrhythmogenic right ventricular cardiomyopathy. Just a
matter of fat? Cardiovasc Pathol. 2005 Jan-Feb;14(1):37-41.

I think you will find some answers to your question. Take in mind that only
a pathologist looking at hundred of hearts and sections per year of
different diseases can reach the experience to differentiate between ARVC and
fatty infiltration or dissociation which is not at all a cardiomyopathy.
Unfortunately there are people which are not pathologists that introduced so
many different terminologies as far as the morphology of the right ventricle
that thay eventually increased the confusion about the real type ARVC.

Best regards

Yours truly

Cristina Basso

Cristina Basso, MD, PhD
Professor, Cardiovascular Pathology
Institute of Pathological Anatomy
University of Padua Medical School
via A. Gabelli, 61
35100 Padova- ITALY

---------------------

Estimado Dr. Lorin de la Grandmaison:

Le sugiero que lea cuidadosamente el trabajo que acabamos de publicar.
Basso C, Thiene G.  Adipositas cordis, fatty infiltration of the right
ventricle, and arrhythmogenic right ventricular cardiomyopathy. Just a
matter of fat? Cardiovasc Pathol. 2005 Jan-Feb; 14(1): 37-41.

Creo que encontrara algunas respuestas a sus preguntas. Tenga en cuenta que
solo un patologo que ha observado cientos de corazones y cortes por anio de
diferentes enfermedades, puede adquirir la experiencia para diferenciar
entre DAVD e infiltracion o disociacion adiposa que no es para nada una
miocardiopatia.
Desdichadamente, existen muchas personas que no son patologos que introducen
tanta terminologia diferente con respecto a la morfologia del ventriculo
derecho, que eventualmente aumentan la confusion acerca del verdadero tipo
de DAVD.

Saludos cordiales,

Cristina Basso

Cristina Basso, MD, PhD
Professor, Cardiovascular Pathology
Institute of Pathological Anatomy
University of Padua Medical School
via A. Gabelli, 61
35100 Padova- ITALY

_______________________________________

>
> English - Spanish
>
> This is a problem that I like to discuss. I hope that Dr Dubner will be
> able to show my slide and discussion based on systematic study of the
> socalled "normal right ventricle"****. In my opinion, in addition to what
> you have said Minor form of myocarditis can produce fibrosis and subsequent
> changes similar to ARVD. This is why we are looking for a more precise
> histologic marker.
> We have seen and published (Kaplan HRS 2004 new journal soon in pubmed...)
> that in Naxos disease there is a definite decrease in RV gap junctions,
> however this feature has not been established in ARVD. However this seems
> to me highly probable because C Guiraudon the pathologist has demonstrated
> the presence desmosomes alteration in ARVD (Europ Heat J). This was for me
> the stating point for the investigation in Naxos which is recessive
> syndromic with a strong phenotypic presentation.
> I also think that in you position in Garches you have the material of
> deaths related to car accident providing an interesting pathologic material
> of  so called "normal right ventricle".
> I join the Fontaliran study on 85 specimen however I am sure that it will
> be transferred properly on the Net.
>
> Guy
>
> SEE THE FILE
> http://www.arvd-symposium.org/files/GradeFat.jpg
>
> SEE THE LECTURE "A mini-Atlas of ARVD Pathology"
> http://www.arvd-symposium.org/lectures.shtml
>
> --------------------
>
> Este es un problema que me gusta discutir. Espero que el Dr. Dubner sea
> capaz de mostrar mis diapositivas y discusion en base al estudio
> sistematico del llamado "ventriculo derecho normal"****. En mi opinion,
> ademas de lo que Ud. ha dicho, la forma menor de miocarditis puede producir
> fibrosis y cambios subsiguientes similares a la DAVD. Esta es la razon por
> la que estamos buscando un marcador histologico mas preciso.
> Hemos visto y publicado (Kaplan HRS 2004 new journal soon in pubmed...) que
> en la enfermedad de Naxos hay una disminucion definitiva en las uniones
> "gap" del VD, sin embargo, esta caracteristica no se ha establecido en la
> DAVD. Sin embargo, a mi me parece que esto es altamente probable porque C.
> Guiraudon, el patologo, ha demostrado la presencia de alteracion
> desmosomica en la DAVD (Europ Heat J). Para mi, este fue el punto de inicio
> para la investigacion de Naxos, que es un sindrome recesivo, con una
> presentacion fuertemente fenotipica.
> Tambien creo que en su lugar en Garches, Ud. tiene el material de muertes
> relacionadas con accidentes de autos, que ofrecen un interesante material
> patologico del llamado "ventriculo derecho normal".
> Me uni al estudio Fontaliran con 85 muestras, sin embargo, estoy seguro de
> que se transferira adecuadamente a Internet.
>
> Guy
>
> VER EL ARCHIVO
> http://www.arvd-symposium.org/files/GradeFat.jpg
>
> VER LA CONFERENCIA "A mini-Atlas of ARVD Pathology"
> http://www.arvd-symposium.org/lectures.shtml
>
>  _______________________________________
>
> > English - Spanish
> >
> > Dear colleagues,
> > Significant fat infiltration of the right ventricle which can be found in
> > autopsy cases always raises the question of a possible underlying ARVD.
> > The difficulty is that such right ventricular lipomatosis can be
> > associated with small foci of fibrosis, especially if there is a small
> > degree atheromatous stenosis, an obesity or a chronic alcohol disease. In
> > such cases, what are the pathological criteria useful to make the
> > differential diagnosis? And do you know if such lipomatosis can induce
> > lethal arrhythmias even if is not a true ARVD?
> >
> > I thank you in advance for your answer.
> >
> > Best regards
> > Geoffroy Lorin de la Grandmaison, MD
> > Department of Pathology and Forensic Medicine, Raymond Poincaré Hospital,
> > Garches, France
> >
> > ---------------------
> >
> > Estimados colegas:
> > La infiltracion adiposa significativa del ventriculo derecho que puede
> > encontrarse en los casos de autopsia siempre hace surgir la cuestion de
> > una posible DAVD subyacente. La dificultad es que tal lipomatosis del
> > ventriculo derecho puede asociarse con pequenios focos de fibrosis,
> > especialmente si hay un grado menor de estenosis ateromatosa, obesidad o
> > alcoholismo cronico. En tales casos, cuales son los criterios patologicos
> > utiles para hacer un diagnostico diferencial? Y saben si tal lipomatosis
> > puede inducir arritmias letales incluso si no es verdadera DAVD?
> >
> > Desde ya les agradezco su respuesta.
> >
> > Saludos cordiales,
> >
> > Geoffroy Lorin de la Grandmaison, MD
> > Department of Pathology and Forensic Medicine, Raymond Poincaré Hospital,
> > Garches, France